Abstract
To determine the reversibility of neurologic abnormalities due to vitamin E (E) deficiency, we prospectively studied the effects of E repletion on 14 children with chronic cholestasis, well-documented E deficiency, and characteristic sural nerve or muscle lesions: Group A(n=2)- both age 20 mo., no neurologic signs; Group B(n=3)- age <3 yrs., with characteristic neurologic signs; Group C(n=9)- ages 5-17 yrs., with neurologic signs. E repletion was achieved by oral (n=4; 60 to 90 IU/kg/day) or intramuscular (n=10;1.0-1.5 IU/kg/day) dl-a-tocopherol. Child neurologists performed serial neurologic exams each 6-12 mo., assigning a neurologic score based on rating each of 12 neurologic signs from 0 (normal) to 3+ (severely abnormal). The mean neurologic scores for each group at 6 month time intervals were (Table):
Conclusion: Vitamin E repletion reverses or prevents neurologic abnormalities when begun prior to age 3 years, whereas only partial improvement occurs when therapy is begun after age 5 years. We recommend surveillance for and treatment of E deficiency prior to age 3 years in children with chronic cholestasis.
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Sokol, R., Guggenheim, M., Iannaccone, S. et al. REVERSIBILITY OF VITAMIN E DEFICIENCY NEUROLOGIC SYNDROME DURING CHRONIC CHILDHOOD CHOLESTASIS. Pediatr Res 18 (Suppl 4), 213 (1984). https://doi.org/10.1203/00006450-198404001-00718
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DOI: https://doi.org/10.1203/00006450-198404001-00718